Universidad Peruana Cayetano Heredia

Altered iPSC-derived neurons' sodium channel properties in subjects with Monge's disease

Mostrar el registro sencillo del ítem

dc.contributor.author Zhao, H. W.
dc.contributor.author Gu, X. Q.
dc.contributor.author Chailangkarn, T.
dc.contributor.author Perkins, G.
dc.contributor.author Callacondo, D.
dc.contributor.author Appenzeller, O.
dc.contributor.author Poulsen, O.
dc.contributor.author Zhou, D.
dc.contributor.author Muotri, A. R.
dc.contributor.author Haddad, G. G.
dc.date.accessioned 2019-02-06T14:53:08Z
dc.date.available 2019-02-06T14:53:08Z
dc.date.issued 2015
dc.identifier.uri https://hdl.handle.net/20.500.12866/5373
dc.description.abstract Monge’s disease, also known as chronic mountain sickness (CMS), is a disease that potentially threatens more than 140 million highlanders during extended time living at high altitudes (over 2500 m). The prevalence of CMS in Andeans is about 15–20%, suggesting that the majority of highlanders (non-CMS) are rather healthy at high altitudes; however, CMS subjects experience severe hypoxemia, erythrocytosis and many neurologic manifestations including migraine, headache, mental fatigue, confusion, and memory loss. The underlying mechanisms of CMS neuropathology are not well understood and no ideal treatment is available to prevent or cure CMS, except for phlebotomy. In the current study, we reprogrammed fibroblast cells from both CMS and non-CMS subjects’ skin biopsies into the induced pluripotent stem cells (iPSCs), then differentiated into neurons and compared their neuronal properties. We discovered that CMS neurons were much less excitable (higher rheobase) than non-CMS neurons. This decreased excitability was not caused by differences in passive neuronal properties, but instead by a significantly lowered Na+ channel current density and by a shift of the voltage-conductance curve in the depolarization direction. Our findings provide, for the first time, evidence of a neuronal abnormality in CMS subjects as compared to non-CMS subjects, hoping that such studies can pave the way to a better understanding of the neuropathology in CMS. en_US
dc.language.iso eng
dc.publisher Elsevier
dc.relation.ispartofseries Neuroscience
dc.rights info:eu-repo/semantics/restrictedAccess
dc.rights.uri https://creativecommons.org/licenses/by-nc-nd/4.0/deed.es
dc.subject Immunohistochemistry en_US
dc.subject Peru en_US
dc.subject Adult en_US
dc.subject Humans en_US
dc.subject Male en_US
dc.subject Young Adult en_US
dc.subject chronic mountain sickness en_US
dc.subject Chronic Disease en_US
dc.subject Cells, Cultured en_US
dc.subject Action Potentials/physiology en_US
dc.subject Altitude Sickness/physiopathology en_US
dc.subject Cell Culture Techniques en_US
dc.subject Fibroblasts/cytology/physiology en_US
dc.subject induced pluripotent stem cells en_US
dc.subject Induced Pluripotent Stem Cells/cytology/physiology en_US
dc.subject Na(+) channel en_US
dc.subject Neural Stem Cells/cytology/physiology en_US
dc.subject Neurogenesis/physiology en_US
dc.subject neurons en_US
dc.subject Neurons/cytology/physiology en_US
dc.subject Patch-Clamp Techniques en_US
dc.subject Sodium Channels/metabolism en_US
dc.title Altered iPSC-derived neurons' sodium channel properties in subjects with Monge's disease en_US
dc.type info:eu-repo/semantics/article
dc.identifier.doi https://doi.org/10.1016/j.neuroscience.2014.12.039
dc.subject.ocde https://purl.org/pe-repo/ocde/ford#3.01.04
dc.relation.issn 1873-7544


Ficheros en el ítem

Ficheros Tamaño Formato Ver

No hay ficheros asociados a este ítem.

Este ítem aparece en la(s) siguiente(s) colección(ones)

Mostrar el registro sencillo del ítem

info:eu-repo/semantics/restrictedAccess Excepto si se señala otra cosa, la licencia del ítem se describe como info:eu-repo/semantics/restrictedAccess

Buscar en el Repositorio


Listar

Panel de Control

Estadísticas